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  • 1
    Electronic Resource
    Electronic Resource
    Pallido-nigro-luysial atrophy with massive appearance of corpora amylacea in the CNS (1981)
    Springer
    Acta neuropathologica 53 (1981), S. 169-172 
    Details
    ISSN: 1432-0533
    Keywords: Pallido-nigro-luysial atrophy ; Combined system degeneration ; Progressive supranuclear palsy ; Corpora amylacea
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of pallido-nigro-luysial atrophy, characterized by symptoms typical of progressive supranuclear palsy, presented (1) severe symmetrical abiotrophic degeneration of the globus pallidus, substantia nigra, and subthalamic nucleus and (2) widespread accumulations of corpora amylacea in the CNS. The former is thought to be consistent with pallidonigro-luysial atrophy and we regard it as a combined system degeneration of both the pallido-nigral and the pallido-luysial systems. The significance of the latter is quite obscure. As far as we know, only three cases of pallido-nigro-luysial atrophy have been reported. However, there is no reported case of this disorder with such widespread and massive accumulations of corpora amylacea as in our case.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00689999
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  • 2
    Electronic Resource
    Electronic Resource
    Nasu-Hakola's disease (membranous lipodystrophy) (1981)
    Springer
    Acta neuropathologica 54 (1981), S. 89-93 
    Details
    ISSN: 1432-0533
    Keywords: Nasu-Hakola's disease ; Membranous lipodystrophy ; Sclerosing leukodystrophy ; Axonal neurofilamentous spheroid ; Neuroaxonal leukoencephalopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsy case of Nasu-Hakola's disease (membranous lipodystrophy) was reported. A 29-yearold Japanese woman whose younger sister had been affected with typical Nasu-Hakola's disease with skeletal and neuropsychiatric sysdromes and membranocystic lesions in the bones developed forgetfulness and lack of initiative. The clinical features were characterized by diminished drive, apathy, euphoria, disturbance of attention, amnestic syndrome, and gait disturbance. The elinical course of her illness was 8 years. The neuropathologic examination revealed marked symmetrical gliosis of the cerebral white matter (sclerosing leukodystrophy) predominantly in the frontal and temporal lobes with slight or moderate demyelination (dissociation glio-myelinique) and widespread axonal changes such as fragmentation and spheroid in the white matter of the cerebral hemisphere, cerebellum, basal ganglia, and brain stem. The ultrastructure of spheroids showed neurofilamentous accumulation. We discussed the importance of axonal changes with regard to the pathogenesis and etiogenesis of the disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00689400
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  • 3
    Electronic Resource
    Electronic Resource
    Presenile dementia with progressive supranuclear palsy tangles and Pick bodies: an unusual degenerative disorder involving the cerebral cortex, cerebral nuclei, and brain stem nuclei (1992)
    Springer
    Acta neuropathologica 84 (1992), S. 128-134 
    Details
    ISSN: 1432-0533
    Keywords: Progressive supranuclear palsy ; Neurofibrillary tangles ; Pick body ; Immunocytochemistry ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Degeneration of heterogeneous systems in the central nervous system, with widespread distribution of argyrophilic neuronal fibrillary inclusions, was found in a patient with presenile dementia. Atrophy was circumscribed in the frontal and temporal lobes. Neuronal loss was severe in the basal ganglia, subthalamic nucleus, and substantia nigra. Immunocytochemical study using anti-phosphorylated tau and anti-ubiquitin antibodies in conjunction with ultrastructural observations revealed two types of inclusions: neurofibrillary tangles (NFTs) of progressive supranuclear palsy (PSP) in the Edinger-Westphal nucleus, locus coeruleus, cerebellar dentate nucleus, inferior olivary nucleus, and posterior horn of the spinal cord; and Pick bodies (PBs) in the atrophied cerebral cortex and red nucleus. PSP-type NFTs and PBs have been demonstrated in a single case for the first time. Despite their pathognomonic significance in certain disorders, we suggest that these inclusions may reflect a form of cytoskeletal disorganization, which is not entirely restricted to a single disease entity.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00311384
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  • 4
    Electronic Resource
    Electronic Resource
    Ultrastructure of 6-aminonicotinamide (6-AN) —Induced lesions in the central nervous system of rats (1978)
    Springer
    Acta neuropathologica 44 (1978), S. 111-119 
    Details
    ISSN: 1432-0533
    Keywords: Pellagra ; 6-Aminonicotinamide ; Cervical gray matter ; Neuronal chromatolysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Ultrastructure of neuronal chromatolysis and other lesions in the gray matter of the cervical cord due to 6-aminonicotinamide (6-AN) administration, an antinicotinamide, to rats, were followed up during a 35-days period. Neuronal chromatolysis which was prominent in the anterior horn cells in the acute stage, was completely recovered from via the temprary hyperchromasia of their cytoplasm. Their axons, however, which form the anterior nerve roots, did not show any particular changes throughout the whole period of the experiment. This evidence suggests that neuronal chromatolysis induced by 6-AN might not be the result of axonal damage, but was due to the direct action of 6-AN on the soma of the anterior horn cells. In addition, necrosis of the internuncial cells, various reactions of glial and mesenchymal elements and the spongy state of the neuropil in the laminae VI and VII of Rexed of the cervical gray matter were observed in the acute stage. They were later repaired by glia. These lesions are usually absent in the central nervous system of human cases with pellagra. It is considered that the findings with 6-AN simulate the central nervous lesions of pellagra in human, but some minor differences in pathology such as presence of severer lesions in glia and other elements in the experimental rats would probably be caused by additional factors such as drastic mode of action, probable difference in catabolism of nicotinamide and 6-AN, and so forth.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00691476
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  • 5
    Electronic Resource
    Electronic Resource
    Distribution of intracytoplasmic acidophilic granule-containing neurons in the human brain (1983)
    Springer
    Acta neuropathologica 60 (1983), S. 145-148 
    Details
    ISSN: 1432-0533
    Keywords: Intracytoplasmic acidophilic granules ; Distribution ; Lewy bodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Using four autopsied brains, we studied the anatomic locations of the intracytoplasmic acidophilic granule (IAG)-containing neurons. These neurons occurred in the hypothalamus, zona incerta, insular cortex, and the other 23 nuclei. However, IAGs were not observed in neurons of the Ammon's horn, thalamus, dentale nucleus, or in Purkinje cells of the cerebellum. The distribution of IAG-containing neurons does not exactly correspond to that of the neuromelanin- or monoamine-forming neurons. There is, however, a striking parallelism between the IAG-containing neurons map and the Lewy bodies map. It is suggested by the superimposition of both maps that IAG-containing neurons may have a certain metabolic relation to the formation of Lewy bodies.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00685360
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  • 6
    Electronic Resource
    Electronic Resource
    Light- and electron-microscopic studies of intracytoplasmic acidophilic granules in the human locus ceruleus and substantia nigra (1982)
    Springer
    Acta neuropathologica 56 (1982), S. 78-80 
    Details
    ISSN: 1432-0533
    Keywords: Intracytoplasmic acidophilic granules ; Melanin-bearing neurons ; Round electron-dense body ; Mitochondrial inclusion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Using the brains of 30 patients with mental and neurologic disorders, we studied the intracytoplasmic acidophilic granules in neurons of the substantia nigra and locus ceruleus by light and electron microscopy. The granules were present in all 30 brains, including those with no recognizable pathologic change, there was no correlation between their appearance and the age, sex, disease of, or the medication received by, the patients. In four electron-microscopically examined brains, we noted many small, round electron-dense bodies in the perikarya and neuronal processes of the substantia nigra and locus ceruleus. The bodies were packed tightly within a double membrane; in shape, size, and distribution in the neuronal cytoplasm, they corresponded to acidophilic granules. Some mitochondrial matrices contained one or more similar, but smaller inclusion bodies; larger bodies pushed aside the mitochondrial cristae. We conclude that the acidophilic granules represent highly developed forms of mitochondrial inclusions.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00691186
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  • 7
    Electronic Resource
    Electronic Resource
    Senile plaque-like structures: observation of a probably unknown type of senile plaque by periodic-acid methenamine silver (PAM) electron microscopy (1989)
    Springer
    Acta neuropathologica 78 (1989), S. 137-142 
    Details
    ISSN: 1432-0533
    Keywords: Senile plaque-like structure ; Periodicacid methenamine silver (PAM) method ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Numerous diffuse senile plaque-like structures (SPLSs) were found in the cerebral cortex from cases with dementia of the Alzheimer type by means of the methenamine-Bodian method. SPLSs varied in shape and size. They were never recognized in the original Bodian, PAS and Congo red preparations, but were positive with anti-β-protein immunostaining and periodic-acid methenamine silver (PAM) methods, which are thought to specifically stain amyloid substance. With PAM electron microscopy, we found sparse aggregations of amorphous, often ramified, structures with fine granular silver deposits in SPLS. Routine electron microscopic examination on the same portion where SPLS were confirmed by PAM electron microscopy revealed amorphous, partially fibrous structures. These structures might be amyloid or amyloid-precursor substance. In SPLSs only a few degenerated neurites and astrocytic processes with glycogen granules were seen. We consider SPLSs to be a kind of senile plaque.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688201
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  • 8
    Electronic Resource
    Electronic Resource
    An early cytoplasmic change before Lewy body maturation: an ultrastructural study of the substantia nigra from an autopsy case of juvenile parkinsonism (1993)
    Springer
    Acta neuropathologica 85 (1993), S. 445-448 
    Details
    ISSN: 1432-0533
    Keywords: Juvenile parkinsonism ; Substantia nigra ; Central pale area ; Early Lewy body
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Neurons containing a central pale area which may possibly represent an early cytoplasmic change before Lewy body maturation were observed in the substantia nigra from a rare autopsy case of juvenile parkinsonism. Ultrastructurally, such neurons exhibited cytoplasmic swelling along with disappearance of the rough endoplasmic reticulum from the central part of the perikaryon. The pale central cytoplasm was replaced by numerous-cored vesicles, mitochondria, ribosomelike granules and a few Lewy body filaments. The relation of this central pale area to the pale body is discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00334457
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  • 9
    Electronic Resource
    Electronic Resource
    Ultrastructural and immunohistochemical study of degenerate neurite-bearing ghost tangles (1992)
    Springer
    Journal of neurology 239 (1992), S. 191-194 
    Details
    ISSN: 1432-1459
    Keywords: Ghost tangles ; Degenerate neurites ; Astrocytes ; Senile plaques ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Some ghost tangles in the brains of Alzheimer patients were accompanied by many small argyrophilic structures which were electron microscopically confirmed to be degenerate neurites. In these ghost tangles, roughly dispersed 15 nm straight and occasional twisted tubules were penetrated by proliferated astrocytic processes. Immunohistochemically, these ghost tangles lost immunoreactivities to anti-NFT, -tau and -ubiquitin antibodies, but were thioflavine-S fluorescent, though antigenicity to β-protein was not proved. This similarity in composition of degenerate neurite-bearing ghost tangles to senile plaques might be induced by the amyloid nature of tubules, which probably provokes the reaction of neuropils.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00839138
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  • 10
    Electronic Resource
    Electronic Resource
    Dentatorubropallidoluysian atrophy: clinicopathological study of dementia and involvement of the nucleus basalis of Meynert in seven autopsy cases (1998)
    Springer
    Acta neuropathologica 96 (1998), S. 502-508 
    Details
    ISSN: 1432-0533
    Keywords: Key words Dentatorubropallidoluysian atrophy ; Dementia ; Nucleus basalis of Meynert ; Neuropathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report concerns a clinicopathological study including a quantitative pathological study on the nucleus basalis of Meynert (nbM) of seven Japanese autopsy cases (four male, three female) of dentatorubropallidoluysian atrophy (DRPLA) with special reference to the clinicopathological correlation of dementia in DRPLA. In each case the pattern of the inheritance was consistent with that of an autosomal dominant trait. The neurological examination revealed that all seven individuals had cerebellar signs. Six patients had epilepsy and choreoathetoid involuntary movement; myoclonus was evident in five patients. Dementia was noted in all seven patients. Degeneration of the globus pallidus (particularly the lateral segment) and of the dentate nucleus was the principal pathological feature. Brain weights at autopsy ranged from 1020 to 1400 g (average 1241 g: male 1320 g, female 1135 g). The quantitative evaluation revealed no significant loss of neurons in the nbM as compared with a control group. There was no clinicopathological correlation between dementia and involvement of the nbM. We suggest that the dementia of DRPLA is due not to the involvement of the nbM, but to – as yet – unidentified pathology elsewhere.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050925
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